A 68-year-old man has many months history of progressive hearing loss, unsteady gait, tinnitus, and facial pain. An MRI scan reveals a tumor at the cerebellopontine angle. Which of the following cranial nerves is this tumor most likely to affect?
An acoustic neuroma is most likely to lead to a palsy of the eighth cranial nerve. Deafness, headache, ataxia, tinnitus, and diplopia are seen, as well as facial paresthesias. Acoustic neuromas represent 5–10% of all intracranial tumors. They develop from Schwann cells and generally grow very slowly. They may be very large before symptoms develop.
A 27-year-old woman presents with acute vision loss and pain in the left eye, but no other symptoms. On examination, she appears well, visual acuity is not possible, and she can only perceive movement and bright light. The direct pupillary reflex is absent but the indirect (consensual) response is normal. The optic disc is edematous. An example of the pathologic changes seen in her optic nerve are shown in Fig. 7–1 a. It shows a plaque of demyelination in the optic nerve as compared to a normal sample in Fig. 7–1 b. Which of the following is the most likely diagnosis?
Visual loss in multiple sclerosis varies from slight blurring to no light perception. Other eye symptoms include diplopia and pain. The classic syndrome of optic or retrobulbar neuritis occurs commonly at some point in the disease, and it is the presenting symptom in 25% of cases.
A 25-year-old man complains of excessive sleepiness during the daytime for years despite adequate nighttime sleep. He has sought medical attention after falling asleep while driving. He is slender and otherwise healthy and on no medications. Which of the following symptoms might he also complain about?
The early age of onset and otherwise good health suggest a diagnosis of narcolepsy, which is usually accompanied by other symptomatology. Hypnagogic hallucinations are almost always visual. They occur most frequently at the onset of sleep, either during the day or at night. They are generally very vivid. Cataplexy is a brief loss of muscle power without loss of consciousness. The patient is fully aware of what is going on. The paralysis may be complete or partial. Automatic behavior with amnesia is a common manifestation of the narcolepsycataplexy syndromes, occurring in 50% of cases. Automatic behavior can be confused with complex partial seizures. Paresthesias are not part of narcolepsy syndrome. Snoring, restless sleep, and morning headache suggest sleep apnea.
A 17-year-old woman presents with symptoms of a fine tremor of her hands. The tremor is best seen when her hands are stretched out. She is not on any medications and reports no alcohol use. Which of the following is the most likely diagnosis?
In hyperthyroidism, neurologic symptoms include tremors of the hands, exophthalmos, lid lag, stare, and muscle weakness. Muscle weakness of hyperthyroidism affects the pelvic girdle and, to a lesser extent, the shoulder girdle. Reflexes are normal or increased, and sensation is normal. It must be differentiated from myasthenia gravis, which may also accompany thyrotoxicosis
A 19-year-old man has had progressive ataxia of gait and great difficulty in running. In the past year, he has developed hand clumsiness. Physical examination reveals pes cavus, kyphoscoliosis, and both cerebellar and sensory changes in the legs. There is a positive family history of Friedreich’s ataxia. Where are the pathologic changes seen in this condition most likely to be found?
This young man has Friedreich’s ataxia, associated with a gene defect on chromosome 9. The pathologic changes are found in the spinal cord tracts. Degeneration is seen in the posterior columns, the lateral corticospinal tract, and the spinocerebellar tracts. Ataxia, sensory loss, nystagmus, reflex changes, clubfeet, and kyphoscoliosis are the characteristic findings. The heart is frequently involved, and cardiac disease is a common cause of death.
A 24-year-old man presents with mild jaundice, tremor, and personality changes. Examination reveals slowness of finger movement, rigidity, and coarse tremor of the outstretched hands. As well there is abnormal slow movement of the tongue and pharynx resulting in a change in speech and occasional difficulty swallowing. He is icteric, the liver span is 10 cm, and no spleen is palpable. Which of the following findings is most likely seen in this condition?
In Wilson’s disease, there is usually a reduction of the serum ceruloplasmin content. Signs and symptoms of injury to the basal ganglia are accompanied by cirrhosis of the liver. Renal involvement is characterized by persistent aminoaciduria. The most common neurologic finding is tremor. The corneal pigmentation (Kayser-Fleischer ring) is the most important diagnostic finding on physical examination. If it is absent, any neurologic findings cannot be ascribed to Wilson’s disease.
A 52-year-old man complains of episodes of severe unilateral, stabbing facial pain that is intermittent for several hours, and then disappears for several days. The pain is describes as “electric shock-like” and only lasts a few seconds. Physical examination of the face and mouth is entirely normal. Which of the following treatments is most effective for this condition?
This patient has trigeminal neuralgia. Carbamazepine (an anticonvulsant drug) is given in doses varying from 600 to 1200 mg/day. Phenytoin has also been used. The two drugs can also be used in combination. Operative procedures include alcohol injection of the nerve or ganglion, partial section of the nerve in the middle or posterior fossa, decompression of the root, and medullary tractotomy. Radiofrequency surgery can destroy pain fibers but spare motor fibers.
A 63-year-old woman develops symptoms of nausea, vomiting, and dizziness, which she describes as a to-and-fro movement of the room like as if she is on a boat. Which of the following findings suggests the vertigo is central in origin?
In central vertigo, the vertigo can be mild and chronic. In peripheral disease, the symptoms are generally more severe, but finite (although often recurrent).
A 30-year-old woman complains of double vision, and easy fatigue with exercise. The fatigue improves with resting, but it is interfering with her work. Examination reveals ptosis and impaired eye movements with normal pupillary response. The double vision is brought out by asking her to look at the ceiling, and after a sustained interval, the eyes slowly drift down. Which of the following is the most likely diagnosis?
In myasthenia gravis, weakness of the facial and levator palpebrae muscles produces a characteristic expressionless face, with drooping of the eyelids. Weakness of the ocular muscles may cause paralysis or weakness of individual muscles, paralysis of conjugate gaze, ophthalmoplegia, or a pattern similar to internuclear ophthalmoplegia. The presence of normal pupillary responses to light and accommodation with weakness of extraocular muscles, levators, and orbicularis oculi is almost completely diagnostic of myasthenia.
A 47-year-old woman presents with increasing headaches and visual changes. On examination, her pupils are normal and reactive to light, the extraocular movements are normal, and there are visual field defects of the outer half in both eyes (bitemporal hemianopsia). Which of the following is the most likely diagnosis?
Adenomas of the pituitary gland constitute approximately 7% of intracranial tumors, with the chromophobic type being the most common. With macroadenomas, some degree of pituitary insufficiency is common, and half the patients have headaches. With microadenomas, the other pituitary functions may be completely normal.
A 45-year-old man presents with weakness and fasciculations in his arms and legs. His cranial nerves are normal, but there is weakness of his left handgrip and right leg quadriceps with loss of muscle bulk. There are obvious fasciculations over the left forearm and right thigh. Tone is increased in the arms and legs and the reflexes are brisk. Which of the following is the most likely diagnosis?
The most common initial symptom of ALS is weakness and wasting of the extremities. The fasciculations can be a very prominent part of the disease. This is rare in other neurologic disorders.
Which of the following is the most likely finding in a 79-year-old woman with Parkinson’s disease?
The characteristic triad in Parkinson’s disease (Tremor, Rigidity, Akinesia) has been expanded to include Postural instability. This forms the mnemonic TRAP. Autonomic instability is also common. Findings on examination also include masklike facies, dysarthria, stooped posture, and abnormal gait.
A 47-year-old man presents to the emergency room with symptoms of dizziness and difficulty walking. He describes his dizziness as a spinning sensation of the room with associated nausea and vomiting. Which of the following findings suggests the vertigo is peripheral in origin?
Tinnitus and deafness may be found in peripheral vertigo, but not central. The nystagmus is usually unidirectional and is never vertical. Visual fixation inhibits vertigo and nystagmus during testing in peripheral vertigo.
A 67-year-old man develops coma over a few minutes. He is unresponsive on arrival to the hospital. He has ataxic respirations and pinpoint, reactive pupils. Oculocephalic reflexes are absent. There is no motor response with noxious stimulation.
Pontine hemorrhage is associated with impaired oculocephalic reflexes and small, reactive pupils. It generally evolves over a few minutes, usually with coma and quadriplegia. The prognosis is poor, and death often occurs within hours.
A 74-year-old woman develops occipital headache, vomiting, and dizziness. She looks unwell, blood pressure 180/100 mm Hg, pulse 70/min, and respirations 30/min. She is unable to sit or walk because of unsteadiness. Over the next few hours, she develops a decline in her level of consciousness.
Cerebellar hemorrhage, when mild, may present with only headache, vomiting, and ataxia of gait. Patients may complain of dizziness or vertigo. The eyes may be deviated to the side opposite the hemorrhage. Nystagmus is not common, but an ipsilateral sixth nerve palsy can occur. This is the only type of intracerebral hemorrhage that commonly benefits from surgical intervention.
A 52-year-old man, with poorly controlled hypertension in the past, presents with increasing headache, confusion, and vomiting. His blood pressure is 230/125 mm Hg, pulse 60/min, respirations 24/min, and there is papilledema. He has no focal deficits.
A 24-year-old man has a history of recurrent throbbing headaches. He suddenly develops mild right-sided weakness. His blood pressure in the past has been normal but is now slightly elevated.
AVMs are more frequently seen in men and, although present from birth, do not usually become symptomatic until later in life. The peak incidence of symptoms is between ages 10 and 30. The headaches can be similar to migraine, or it can be more diffuse. It can also present with seizure or rupture. Hemorrhage can be massive or minimal when rupture does occur.
A 20-year-old man develops fatigue and severe muscle weakness of his limbs usually after eating a large meal. He is diagnosed with familial periodic paralysis.
The most common familial periodic paralysis syndrome is usually associated with low potassium, but there are less common forms characterized by high or normal potassium. It is characterized by recurrent attacks of weakness or paralysis of the somatic musculature, with loss of the deep tendon reflexes. Preventive therapy includes potassium supplementation and possibly a low carbohydrate, low salt, high potassium diet. Imipramine and acetazolamide are said to be useful in acute attacks.
A 27-year-old woman complains of double vision and fatigue at the end of the day. Further history reveals difficulty in chewing food, and some weakness in climbing stairs. She has stopped running because of easy leg fatigue and leg weakness. The symptoms improve with resting. On examination, there is weakness of the eyelids, masticatory muscles, and thigh flexors. Her handgrip decreases with repetitive action. There is no sensory abnormality, and reflexes are normal. Which of the following is the most likely diagnosis?
A 20-year-old man suffered a significant closed head injury after a diving accident. He is left with minor memory impairment but makes a full recovery. A few months later, he has a witnessed loss of consciousness with some arm and leg twitching. After the event, he is disoriented for 2 hours and then gradually returns to baseline. There was no incontinence or tongue biting, and his examination is normal. Which of the following is the most likely diagnosis?
This man had a generalized posttraumatic seizure. The prolonged confusion after the event suggests seizure rather than syncope. In the majority of cases, seizures do not develop until several months after the injury, 6–18 months being the most common interval. The more severe the injury, the greater the likelihood of seizures. For severe injuries, some authorities recommend prophylactic anticonvulsants for 1–2 years. There is no firm evidence for this, however.
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