Balint-Holmes syndrome combining paralysis of visual fixatio

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NEUROLOGY SYNDROMES

Balint's syndrome
Also known as: Balint-Holmes syndrome
Description: A syndrome combining paralysis of visual fixation, optic ataxia, and impairment of visual fixation. It is marked by inability to execute voluntary movement in response to visual stimuli. Despite normal field of view and normal acuity the patients perceives only one object, from which he can hardly move his eyes, while all other objects are not recognised. A rare disorder of oculomotor function due to bilateral lesions of the parietal and occipital lobes.
Rabbit syndrome
A rare neuroleptic-induced form of parkinsonism usually manifested as 4–6 Hz, rhythmic discharges involving the jaw, perinasal, and perioral musculature; so-named because the movements mimic those seen with the chewing movements of a rabbit.
Rabbit syndrome is an uncommon, tongue-sparing, orofacial movement disturbance, rapid and regular in nature and associated with prolonged use of neuroleptics. Two cases reports illustrate how anticholinergic agents successfully treat the disorder. The syndrome is distinguished from tardive dyskinesia, which tends to be exacerbated by anticholinergic agents.
Kennedy's syndrome
Also known as:Kennedy's phenomenon, Foster Kennedy's syndrome, Gowers-Paton-Kennedy syndrome.
Description: Condition characterised by unilateral ipsilateral atrophy with contralateral papilloedema, central scotoma, and anosmia (absence of the sense of smell) usually due to a frontal lobe tumour or a meningioma of the optic nerve. There may be headache, dizziness, vertigo; occasionally, forceful vomiting, psychic changes (moria), memory loss.
Melkersson-Rosenthal syndrome
A rare neurological disorder characterized by recurring facial paralysis, swelling of the face and lips (usually the upper lip), and the development of folds and furrows in the tongue. Onset is in childhood or early adolescence. After recurrent attacks (ranging from days to years in between), swelling may persist and increase, eventually becoming permanent. The lip may become hard, cracked, and fissured with a reddish-brown discoloration. The cause of Melkersson-Rosenthal syndrome is unknown, but there may be a genetic predisposition.
Granulomatous cheilitis is a chronic swelling of the lip due to granulomatous inflammation. Miescher cheilitis is the term used when the granulomatous changes are confined to the lip. Miescher cheilitis is generally regarded as a monosymptomatic form of the Melkersson-Rosenthal syndrome, although the possibility remains that these may be 2 separate diseases. Melkersson-Rosenthal syndrome is the term used when cheilitis occurs with facial palsy and plicated tongue.
Melkersson-Rosenthal syndrome is occasionally a manifestation of Crohn disease or orofacial granulomatosis.
Horner Syndrome
Horner syndrome results from an interruption of the sympathetic nerve supply to the eye, and is characterized by the classic triad of miosis (ie, constricted pupil), partial ptosis, and loss of hemifacial sweating (ie, anhidrosis).
A syndrome associated with defective sympathetic innervation to one side of the face, including the eye. Clinical features include MIOSIS; mild BLEPHAROPTOSIS; and hemifacial ANHIDROSIS (decreased sweating)(see HYPOHIDROSIS). Lesions of the BRAIN STEM; cervical SPINAL CORD; first thoracic nerve root; apex of the LUNG; CAROTID ARTERY; CAVERNOUS SINUS; and apex of the ORBIT may cause this condition. It is also known by the names Bernard-Horner syndrome or oculosympathetic palsy.
Aphasia
Aphasia is a complex acquired neurogenic disorder in which there are problems with speaking or speech is lost. It is due to an injury to certain areas of the brain. There are many forms and degrees of aphasia. For example, a patient with aphasia may be able to speak but not to form words that can be understood. The patient may be able to understand speech and writing and form thoughts, but not be able to speak. Aphasia may be the result of a severe head injury, lack of oxygen, or stroke. It is sometimes short term, as when a swelling in the brain goes down and language returns. Constant hard work and practice by the patient and the patient's family have helped to restore normal speaking ability. There are a variety of differing types of aphasia classified by the patient's deficit in language ability. Broca's aphasia is characterized by an almost telegraphic speech pattern in which the words that are uttered are short and often agrammatic, while comprehension remains comparatively intact. Wernicke's aphasia is characterized by fluent speech, but poor comprehension. Global aphasia is characterized by comprehension deficits and non-fluent speech. Transcortical Motor Aphasia is similar to Broca's aphasia except the ability to repeat is intact. Transcortical Sensory Aphasia is similar to Wernicke's aphasia but the ability to repeat spoken language is spared. Conduction aphasia is also known as disconnection syndrome because it is a lesion that disconnects Broca's area from Wernicke's area via the arcuate fasciculus and is characterized by a comparatively reduced ability to repeat spoken language. Anomic aphasia is characterized by an inability to come up with the names of common objects.
The traditional classification scheme includes eight types of aphasia :
Broca's aphasia, also called motor aphasia, results from damage to the front portion or frontal lobe of the language-dominant area of the brain. Individuals with Broca's aphasia may be completely unable to use speech (mutism) or may be able to use single-word statements or even full sentences, though these sentences may require a great deal of effort to construct. Small words, such as conjunctions (and, or, but) and articles (the, an, a), may be omitted, leading to a "telegraph" quality in their speech. Hearing comprehension is usually not affected, so they are able to understand other people's speech and conversation and can follow commands. Often, they may experience weakness on the right side of their bodies, which can make it difficult to write. Reading ability is impaired, and they may have difficulty finding the right word when speaking. Individuals with Broca's aphasia may become frustrated and depressed because they are aware of their language difficulties.
Wernicke's aphasia is caused by damage to the side portion or temporal lobe of the language-dominant area of the brain. Individuals with Wernicke's aphasia speak in long, uninterrupted sentences; however, the words used are frequently unnecessary or even made-up. They have a great deal of difficulty understanding other people's speech, sometimes to the point of being unable to understand spoken language at all. Reading ability is diminished, and although writing ability is retained, what is written may be abnormal. No physical symptoms, such as the right-sided weakness seen with Broca's aphasia, are typically observed. Also, in contrast to Broca's aphasia, individuals with Wernicke's aphasia are not aware of their language errors.
Global aphasia is caused by widespread damage to the language areas of the left hemisphere. As a result, all basic language functions are affected, but some areas may be more affected than others. For example, an individual may have difficulty speaking but may be able to write well. The individual may experience weakness and loss of feeling on the right side of their body.
Conduction aphasia, also called associative aphasia, is rather uncommon. Individuals with conduction aphasia are unable to repeat words, sentences, and phrases. Speech is fairly unbroken, although individuals may frequently correct themselves and words may be skipped or repeated. Although able to understand spoken language, it may also be difficult for the individual with conduction aphasia to find the right word to describe a person or object. The impact of this condition on reading and writing ability varies. As with other types of aphasia, right-sided weakness or sensory loss may be present.
Anomic or nominal aphasia primarily influences an individual's ability to find the right name for a person or object. As a result, an object may be described rather than named. Hearing comprehension, repetition, reading, and writing are not affected, other than by this inability to find the right name. Speech is fluent, except for pauses as the individual tries to recall the right name. Physical symptoms are variable, and some individuals have no symptoms of one-sided weakness or sensory loss.
Transcortical aphasia is caused by damage to the language areas of the left hemisphere outside the primary language areas. There are three types of aphasia: transcortical motor aphasia, transcortical sensory aphasia, and mixed transcortical aphasia. All of the transcortical aphasias are distinguished from other types by the individual's ability to repeat words, phrases, or sentences. Other language functions may also be impaired to varying degrees, depending on the extent and particular location of brain damage.
Thyrotoxic Myopathy
Thyrotoxic myopathy is a neuromuscular disorder that may accompany hyperthyroidism (Grave’s disease, caused by overproduction of the thyroid hormone thyroxine). Symptoms may include muscle weakness, wasting of the pelvic girdle and shoulder muscles, fatigue, and heat intolerance.
Thyrotoxic myopathy is a neuromuscular disorder that occurs due to overproduction of thyroid hormone and is characterized by excessive fatigability, muscle wasting and weakness. It mainly affects muscles of the shoulder, hips and hands. The adverse effects of thyroid hormone on the structure and function of muscles gives rise to this myopathy. Although diagnosis can be tricky, this disorder is reversible with appropriate treatment.
Thyrotoxic myopathy is known by several other names like hyperthyroid myopathy, Graves and Basedow's myopathy or Basedow paraplegia. It was first recognized in the early nineteenth century by Graves and Von Basedow as occurring infrequently in severe hyperthyroidism. In the middle of the twentieth century, researchers found that up to 80% of hyperthyroid patients manifested at least some degree of muscle weakness and this was confirmed on electromyographic studies.
Gerstmann's syndrome
Also known as:Gerstmann syndrome II, Syndrome de Gerstmann (French), Gerstmann-Badal syndrome
Description:Disease of the association area of the dominant parietal lobe of the brain with characteristic clinical picture. Symptoms are finger agnosia (lacking or impaired ability to describe the fingers), agraphia (lacking or impaired ability to write), right-left disorientation, dysphragia; dyscalculia/acalculia (lack of ability to calculate). The patient has difficulty in recognizing, naming, selecting, and differentiating the fingers of either of his hands or those of others. In addition, there is inability to recognize the sides of his body, to write words or sentences, and to perform even simple calculations. Orientation for place and person is not affected, but time orientation may be somewhat disturbed. The general psychic condition of the patient is good. It is debated whether this is really an independent syndromic entity.
Gerstmann's syndrome is a neurological disorder characterized by four primary symptoms: a writing disability (agraphia or dysgraphia), a lack of understanding of the rules for calculation or arithmetic (acalculia or dyscalculia), an inability to distinguish right from left, and an inability to identify fingers (finger agnosia). The disorder should not be confused with Gerstmann-Strä ussler-Scheinker disease, a type of transmissible spongiform encephalopathy.
In adults, the syndrome may occur after a stroke or in association with damage to the parietal lobe. In addition to exhibiting the above symptoms, many adults also experience aphasia, (difficulty in expressing oneself when speaking, in understanding speech, or in reading and writing).
There are few reports of the syndrome, sometimes called developmental Gerstmann's syndrome, in children. The cause is not known. Most cases are identified when children reach school age, a time when they are challenged with writing and math exercises. Generally, children with the disorder exhibit poor handwriting and spelling skills, and difficulty with math functions, including adding, subtracting, multiplying, and dividing. An inability to differentiate right from left and to discriminate among individual fingers may also be apparent. In addition to the four primary symptoms, many children also suffer from constructional apraxia, an inability to copy simple drawings. Frequently, there is also an impairment in reading. Children with a high level of intellectual functioning as well as those with brain damage may be affected with the disorder.
Gerstmann syndrome is a cluster of neurological symptoms that includes difficulty writing (dysgraphia or agraphia), difficulty with arithmetic (dyscalculia or acalculia), an inability to distinguish left from right, and difficulty identifying fingers (finger agnosia).
Two types of Gerstmann syndrome have been identified: an acquired form that occurs in adults who have suffered brain injury through stroke or trauma, and a developmental form that has been noted in children.
The brain area that seems to be primarily responsible for the deficits seen in Gerstmann syndrome appears to be the parietal lobe, which is located behind the frontal lobe. Current research has not identified a tendency for the developmental form of Gerstmann syndrome to be inherited.
Although both adults and children with Gerstmann syndrome may have considerable impairment, they do not necessarily have abnormal intelligence
Shy-Drager Syndrome
Multiple system atrophy with autonomic failure, also called Shy-Drager syndrome, is a progressive disorder of the central and autonomic nervous systems. The disorder is characterized by postural hypotension--an excessive drop in blood pressure which causes dizziness or momentary blackouts upon standing or sitting up. There are 3 types of Shy-Drager syndrome: Parkinsonian-type which may include symptoms of Parkinson's disease such as slow movement, stiff muscles, and mild tremors; cerebellar-type which may include problems such as loss of balance and the tendency to fall; and combination-type which may include symptoms of both types 1 and 2. Parkinsonian symptoms and symptoms of autonomic dysfunction such as constipation and sexual impotence in males predominate early in the course of the disease. Constipation may be unrelenting and hard to manage in some patients. Shy-Drager may be difficult to diagnose in the early stages; however, within a year of onset most patients develop postural hypotension. For the majority of patients, blood pressure is unstable--often fluctuating up and down--and causes severe headaches. Other symptoms may also develop, such as generalized weakness, double vision and/or other vision disturbances, impairment of speech, sensory changes, difficulties with breathing and swallowing, irregularities in heart beat, inability to sweat, and diarrhea.
A progressive neurodegenerative condition of the central and autonomic nervous systems characterized by atrophy of the preganglionic lateral horn neurons of the thoracic spinal cord, which differentiates this condition from other forms of idiopathic orthostatic hypotension (HYPOTENSION, ORTHOSTATIC). This disease is generally considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Affected individuals present in the fifth or sixth decade with orthostasis and bladder dysfunction; and later develop FECAL INCONTINENCE; anhidrosis; ATAXIA; IMPOTENCE; and alterations of tone suggestive of basal ganglia dysfunction.
Millard-Gubler syndrome
Also known as: Gubler’s hemiplegia, Gubler’s paralysis, Gubler’s syndrome, Gubler-Millard syndrome, Millard’s syndrome, Millard-Gubler-Foville syndrome, Raymond's syndrome, Raymond-Foville syndrome.
Description:A syndrome of unilateral softening of the brain tissue arising from obstruction of the blood vessels of the pons, involving the sixth and seventh cranial nerves and fibres of the corticospinal tract, and associated with paralysis of the abducens and facial nerves and contralateral hemiplegia of the extremities. The muscles of the ipsilateral side of the face are paralysed, and the ophthalmologic characteristics are diplopia, internal strabismus, and loss of power to rotate eye outward.
If there is also paralysis of inward movement of the eye in attempting to look toward the side of the lesion, the condition is known as Foville syndrome.
Millard Guber Syndrome results from occlusion of the penetrating branches of the basilar artery in the pons. It is characterised by:
lateral rectus palsy - cranial VI
ipsilateral facial paralysis - cranial VII
contralateral hemiplegia
The syndrome is often accompanied by contralateral sensory loss - light touch and proprioception - due to medial leminiscal damage. However, this is not part of the defined syndrome.
Anton Syndrome
Cerebral hemisphere: Bilateral occipital lobes
Posterior cerebral artery: Bilateral; Basilar artery: Top of the basilar
Signs & Symptoms:*Side: Manifestation: Comments: B Visual loss - bilateral ; N Unawareness or denial of blindness
May have visual hallucinations.
Balint Syndrome
Cerebral hemisphere: Bilateral parietal-occipital lobes
Vascular: Posterior cerebral artery: Bilateral
Signs & Symptoms:
*Side: Manifestation: Comments:
B Loss of voluntary but not reflex eye movements
B Optic ataxia - poor visual-motor coordination
B Asimultagnosia - inability to understand visual objects
Claude Syndrome
Midbrain: Tegmentum
Vascular:Posterior cerebral artery
Signs & Symptoms:
*Side: Manifestation: Comments:
C Ataxia - arm and leg
Oculomotor palsy with contralateral tremor and ataxia.
Medial medullary syndrome
Dejerine Syndrome
Medulla: Medial medulla
Vascular:Vertebral artery: Anteromedial artery
Anterior spinal artery: Anteromedial artery
Basilar artery
Signs & Symptoms:
*Side: Manifestation: Comments:
C Weakness - upper and lower extremity Pyramidal tract
C Hemisensory loss - vibration and proprioception Medial lemniscus
I Tongue weakness +/- atrophy Cn 12 nucleus
Rare stroke syndrome (<1% of vertebrobasilar strokes, Bassetti et al., 1994). Medial medullary infarct is associated with clinical triad of ipsilateral hypoglossal palsy, contralateral hemiparesis, and contralateral lemniscal sensory loss. Variable manifestations may include isolated hemiparesis, tetraparesis, ipsilateral hemiparesis, I or C facial palsy, ataxia, vertigo, nystagmus, dysphagia. Palatal and pharyngeal weakness rare in pure MMI, common in lateral medullary infarct.
Thalamic pain syndrome
Dejerine-Roussy syndrome
Thalamus
Vascular:Posterior cerebral artery: Pentrating branches to thalamus
Signs & Symptoms:
*Side: Manifestation: Comments:
C Hemisensory loss - all modalities
C Hemi-body pain
Inferior medial pontine syndrome
Foville Syndrome
Anatomy : Pons: Unilateral lesion in the dorsal pontine tegmentum in the caudal third of the pons
Vascular:Basilar artery: Paramedian branches ; Basilar artery: Short circumferential arteries
Signs & Symptoms:
*Side: Manifestation: Comments:
C Weakness - upper and lower extremity Corticospinal tract
I Weakness - face - entire side VII nucleus / fascicle
I Lateral gaze weakness PPRF or CN VI nucleus
Foville Syndrome, Inferior medial pontine syndrome
Unilateral lesion in the dorsal pontine tegmentum in the caudal third of the pons.
1. Contralateral hemiplegia (with facial sparing) due to corticospinal tract involvement
2. Ispilateral peripheral-type facial palsy, due to cranial nerve VII nucleus/fascicle involvement.
3. Inability to move the eyes conjugately to the ipsilateral side due to paramedian pontine reticular formaiton and/or abducens nerve nucleus invovlement. That is, patient is unable to look toward the lesion.
Note: this is also called Millard-Gubler syndrome.
Gerstmann Syndrome
Anatomy : Cerebral hemisphere: Dominant parietal lobe
Vascular: Middle cerebral artery
Signs & Symptoms:
*Side: Manifestation: Comments:
N Agraphia (inability to write)
N Acalculia (inability to calculate)
N Right-left confusion
N Finger agnosia (inability to recognize fingers)
N Ideomotor apraxia May be associated
Lateral pontine syndrome
Marie-Foix Syndrome
Vascular:Basilar artery: Long circumferential branches ; Anterior inferior cerebellar artery
Signs & Symptoms:
Side: Manifestation: Comments:
I Ataxia - arm and leg Cerebellar tracts
C Weakness - upper and lower extremity Corticospinal tracts
C Hemisensory loss - pain and temperature Spinothalamic tract
Marie-Foix Syndrome
Lesion in the lateral pons, including the middle cerebellar peduncle.
1. Ipsilateral cerebellar ataxia due to involvement of cerebellar tracts
2. Contralateral hemiparesis due to corticospinal tract involvement
3. Variable contralateral hemihypesthesia for pain and temperature due to spinothalamic tract involvement.
Ventral pontine syndrome
Millard-Gubler Syndrome
Anatomy : Pons: Basis pontis and fascicles of CN VI amd VII
Vascular: Basilar artery: Short circumferential branches ; Basilar artery: Paramedian branches
Signs & Symptoms:
Side: Manifestation: Comments:
C Weakness - upper and lower extremity Pyramidal tract
I Lateral gaze weakness CN VI
I Weakness - face - entire side CN VII
Millard-Gubler Syndrome
A unilateral lesion of the ventrocaudal pons may invovle the basis pontis and the fascicles of cranial nerves VI and VII. Symptoms include:
1. Contralateral hemiplegia (sparing the face) due to pyramidal tract involvement
2. Ipsilateral lateral rectus palsy with diplopia that is accentuated when the patient looks toward the lesion, due to cranial nerve VI involvement.
3. Ipsilateral peripheral facial paresis, due to cranial nerve VII involvement
Ventral pontine syndrome
Raymond Syndrome
Anatomy : Pons: Ventral medial pons
Vascular: Basilar artery: Paramedian branches
Signs & Symptoms:
Side: Manifestation: Comments:
I Lateral gaze weakness CN VI
C Weakness - upper and lower extremity Pyramidal tract
Raymond Syndrome (Alternating abducens hemiplegia) A unilateral lesion of the ventral medial pons, which affects the ipsilateral abducens nerve fascicles and the corticospinal tract but spares cranial nerve VII.
1. Ipsiplateral lateral rectus paresis, due to cranial nerve VI involvement
2. Contralateral hemipegia, sparing the face, due to pyramidal tract involvement.
Lateral medullary syndrome
Wallenberg Syndrome
Vascular:Vertebral artery: Distal branchesVertebral artery: Superior lateral medullary artery; Posterior inferior cerebellar artery: Less common than vertebral
Signs & Symptoms:
Side: Manifestation: Comments:
I Sensory loss - face - pain and temperature CN 5 nucleus
I Facial pain CN 5 nucleus
I Ataxia - arm and leg Restiform body, cerebellum
I Gait ataxia Restiform body, cerebellum
I Nystagmus Vestibular nucleus
I Nausea / vomiting Vestibular nucleus
I Vertigo Vestibular nucleus
I Horseness Nucleus ambiguus
I Dysphagia Nucleus ambiguus
I Horner syndrome Descending sympathetics
C Hemisensory loss - pain and temperature Spinothalamic tract
N Hiccups
Weber Syndrome
Anatomy : Midbrain: Base
Vascular: Posterior cerebral artery: Penetrating branches to midbrain
Signs & Symptoms:
Side: Manifestation: Comments:
C Weakness - upper and lower extremity
Corticospinal tract
I Lateral gaze weakness CN 3
Locked-in Syndrome
Anatomy : Pons: Bilateral ventral pons
Vascular: Basilar artery
Signs & Symptoms:
Side: Manifestation: Comments:
B Weakness - upper and lower extremity Quadriplegia: bilateral cortical spinal tracts
B Weakness - face - entire side Bilateral corticobulbar tracts
N Lateral gaze weakness Bilateral fascicles of CN VI
N Dysarthria Bilateral corticobulbar tracts
Bilateral ventral pons lesions (iscemic or hemorrhagic) may result in this deefferented state, with preserved consciousness and sensation, but paralysis of all movements except vertical gaze and eyelid opening.
1. Quadriplegia due to bilateral corticospinal tract involvement
2. Aphonia due to corticobulbar tract involvement to lower cranial nerve nuclei
3. Occasionally, impairment of horizontal eye movements due to bilateral involvement of the fasciclesof cranial nerve
4. Reticular formation is spared, so the patient is typically fully awake. The supranuclear ocular motor pathways lie dorsally, so that vertical eye movements and blinking are intact.
Benedict Syndrome
Infarctions that occur in the brain stem which is comprised of the midbrain, pons, and medulla. There are several named syndromes characterized by their distinctive clinical manifestations and specific sites of ischemic injury.

Named Brainstem Syndromes
Eponym Site Cranial Nerves Tracts Signs Usual Cause
Weber Base of Midbrain III Corticospinal Oculomotor palsy with crossed hemiplegia Vascular, tumor
Claude Midbrain tegmentum III Red nucleus and Brachium Conjunctivum Oculomotor palsy with contralateral cerebellar ataxia and tremor Vascular, tumor
Benedict Midbrain tegmentum III Red nucleus, corticospinal tract, brachium conjunctivum Oculomotor palsy, contralateral cerebellar ataxia, corticospinal signs Vascular, tuberculoma, tumor
Nothnagel Midbrain tectum Unilateral or bilateral III Superior cerebellar peduncles Ocular palsies, paralysis of gaze, cerebellar ataxia Tumor
Parinaud Dorsal Midbrain Paralysis of upward gaze and accommodation, fixed pupils, retraction nystagmus Pinealoma, hydrocephalus
Millard-Gubler and Raymond-Foville Base of Pons VII and sometimes VI Corticospinal tract Facial and 6th palsy, contralateral hemiplegia, sometimes gaze palsy Vascular,tumor
Avellis Medulla tegmentum X Spinothalamic, sometimes pupillary fibers Paralysis of soft palate and vocal cord and contralateral hemianesthesia Infarct or Tumor
Jackson Medulla Tegmentum X,XII Corticospinal Avellis plus ipsilateral tongue Infarct or Tumor
Wallenberg Medulla, lateral tegmentum Spinal V,IV,X,XI Lateral STT,Descending Pupil fibers, Spinocerebellar and olivocerebellar tracts Ipsi V, IV, X, XI palsy, Horner's, cerebellar ataxia. Contra pain and temp Vascular - Pica or vertebral